The undetermined form of Chagas' heart disease: concept and forensic implications

The undetermined form of Chagas' disease is diagnosed in asymptomatic subjects with a positive blood test for Chagas' disease, normal resting electrocardiogram, chest X-ray, barium esophageal and large bowel radiological studies. Other investigation methods are not recommended for identification of other organs damage lesions in this phase of the disease. When other methods of investigation were employed, cardiac and digestive abnormalities of small magnitude were detected without prognostic implications. These findings do not warrant frequent examinations of patients with undetermined form of the disease except for the electrocardiogram or if the patients report other clinical manifestations. The benign course of the disease does not preclude ability to work and the subjects should be considered apt for work in any profession.     

Mental health in the north west region of England: associations with deprivation

OBJECTIVES: To evaluate the prevalence, clinical and radiological characteristics, association with HLA B27 in a subgroup of patients with inflammatory bowel disease (IBD) and subclinical sacroileitis. The sensitivity of the diagnostic criteria for spondyloarthropathy in this group of patients is evaluated. MATERIALS AND METHODS: All patients with inflammatory bowel disease attending an outpatient gastroenterology unit from January 1994 to June 1994 were recruited. A total of 62 patients with IBD and without clinical evidence of axial involvement were included in the study. The demographic, clinical, and radiological characteristics were collected. The radiological examination included PA and lateral views for the dorso-lumbar, and three views (Ferguson, right and left oblique views) for the sacroiliac joints. Films were interpreted by three independent readers. The HLA B27 allele distribution was analyzed in the 62 patients and in 80 healthy controls. The modified New York criteria, Amor criteria, and European Spondyloarthropathy Study Group criteria were evaluated. Patients were prospectively followed for two years with the same initial protocol. The statistical management of data was performed with the information program SPSS/PC. RESULTS: Fifteen cases of silent sacroileitis were detected, and most of them were grade 2 unilateral sacroileitis. There was no correlation between sacroileitis and IBP type, extradigestive symptoms, disease duration, sex, or peripheral arthritis. The frequency of HLA B27 in the sacroileitis group was 20% (p < 0.05). During the two-year follow-up period none of these cases has changed from diagnostic category. The sensitivity of diagnostic the criteria for spondyloarthropathy was low in these patients (40%-53%). CONCLUSIONS: A high frequency of asymptomatic sacroileitis in patients with IBD was detected. We propose the term Silent Axial Arthropathy to define this category of patients and, as with other authors, we consider this is a third form of rheumatic syndrome in IBD, different from the classical forms of peripheral arthritis and ankylosing spondylitis.     

Cardiac rupture with dobutamine stress echocardiography

Dobutamine stress echocardiography is a safe method for the evaluation of coronary artery disease, myocardial function, and viability. The potential for cardiac rupture exists. Although clinical and electrocardiographic criteria may be helpful in identifying those patients at risk for cardiac rupture, the criteria are neither sensitive nor specific enough to exclude patients or make recommendations regarding which patients should undergo dobutamine stress echocardiography in the post-infarction period. Physicians must be aware of the possibility and be prepared to treat cardiac rupture when performing dobutamine stress echocardiography.     

Pregnancy, puerperium and cerebrovascular disease

Six patients who presented cerebrovascular disease during puerperium or pregnancy were studied. One of them presented hemorrhagic stroke caused by intracerebral bleeding due to pre-eclampsia. Three patients presented ischemic stroke, they all had positive serologic reactions for Chagas' disease, but only two of them had clinical cardiopathy; one of these patients had anticardiolipin antibody. The other two patients presented cerebral venous thrombosis of sagittal sinus. Both were smokers and one of them used oral contraceptive. We emphasize the importance of clinical investigation to seek for the common causes of cerebrovascular disease in young people, with special attention to Chagas disease in Brazil.     

Ventricular tachycardia revealing viral myocarditis

BACKGROUND: The first diagnostic hypothesis in a middle-aged patient presenting with inaugural ventricular tachycardia would be coronary artery disease. If the work-up lacks arguments for this etiology, other cardiac conditions may be involved (dilated cardiopathy, hypertrophic cardiopathy, valve disease arrhythmogenic dysplasia, long QT...). CASE REPORT: A 52-year-old male patient was referred for inaugural ventricular tachycardia. The initial work-up including echocardiography, coronography and the electrophysiologic study provided no explanation. The ventricular tachycardia was later attributed to viral myocarditis. DISCUSSION: Viral myocarditis should always be entertained in patients with unexplained ventricular tachycardia, particular if a viral context is present. In such cases, antibody-labeled scintigraphy is the choice exploration. This noninvasive technique provides determining diagnostic information and helps orient patient management.     

Chronic non-specific parotid sialadenitis

Electrocardiographic and clinical characteristics are currently used as diagnostic criteria for the long QT-syndrome. In borderline electrocardiographic findings associated with unclear syncope, it is often difficult to ensure or exclude long QT-syndrome. Schwartz and coworkers therefore created a point system as a guide in clinical decision making. In recent years genetic diagnostics have entered the arena of long-QT assessment. Aside from new insights into the pathophysiology of the long QT-disorder, it is expected that genetic diagnostics will offer substantial help to ascertain long QT-syndrome in patients with borderline electrocardiographic and clinical findings and improve risk stratification in long-QT family members. Performing linkage analysis, coupling of autosomal-dominant congenital long QT-syndrome (Romano-Ward Syndrome) to chromosomes 11 (LQT1/11p15.5), 3 (LQT3/3p21), 7 (LQT2/7q35), and 4 (LQT4/4q25-27) was demonstrated. More recently, the disease genes in long QT-syndrome 1, 2, and 3 could be identified. Analysis of the base-pair sequence allowed detection of several different mutations in different families illustrating genetic heterogeneity. Aside from diagnostic aspects, molecular genetics may also guide pharmacological therapy by identifying the specific ion-channel disorder leading to QT-prolongation and sudden death.     

Evaluation of quality of life in sarcoidosis patients

The Western blot method, using antigens from epimastigote forms of the Trypanosoma cruzi Y strain, was evaluated for the confirmatory diagnosis of Chagas' disease. Serum samples were obtained from 136 chagasic patients (Group I), 23 patients with inconclusive serologic results for Chagas' disease (Group II), 53 patients with other diseases (Group III), and 50 healthy individuals (Group IV). The Western blot results for Group I gave a confirmatory diagnosis of Chagas' diseases in 118 (86.80%), an indeterminate pattern of reactivity in 16 (11.76%), and a negative pattern in only two (1.47%). Of the samples from Groups II, III and IV, none had a positive result in the Western blot; 60.86%, 52.83%, and 16.0%, respectively, showed indeterminate results; and 39.13%, 47,16% and 84,0%, respectively, showed negative results. The Western blot method showed a sensitivity of 86.60%, a specificity of 100.0%, a positive predictive value of 100.0%, and a negative predictive value of 73.50%, and the concordance coefficient kappa was high (0.7789). The results suggest that the previous serologic results for Chagas' disease could be confirmed by Western blot for the detection of specific antibodies to T. cruzi antigenic fractions, which may reduce the medical, legal, and social consequences of an inconclusive serologic result for Chagas' disease and also underscore the need for additional studies for continued efforts in the development of an ideal standard confirmatory test for Chagas' disease.     

Citrullinemia: management and clinical course. Apropos of a familial case

The authors report two cases of citrullinemia in siblings which add to 68 observations from the literature. They overview the clinical presentation, diagnosis and therapeutic management of the disease. The prognosis of severe neonatal form remains poor but an early adequate management may contribute to an acceptable outcome.     

Perioperative assessment and management of risk from coronary artery disease

PURPOSE: To summarize available evidence on preoperative cardiac risk stratification so that the internist may 1) use clinical and electrocardiographic findings to stratify a patient's perioperative risk for myocardial infarction and death; 2) decide which tests provide useful additional risk-related information; and 3) understand the benefits, risks, and evidence surrounding the decision to undertake coronary revascularization before elective noncardiac surgery. DATA SOURCES: A MEDLINE search and review of the reference lists of identified articles. Sensitivities, specificities, and likelihood ratios for diagnostic tests were calculated, and a quality rating for study methods was applied. DATA EXTRACTION: Myocardial infarction and mortality were the major outcomes considered, and a quality rating for study methods was applied. DATA SYNTHESIS: Clinical and electrocardiographic findings, organized by multivariate prediction indices, accurately identify patients as having low, intermediate, or high risk for myocardial infarction or death. Pharmacologic stress imaging with thallium or echocardiography probably improves risk stratification for intermediate-risk patients having vascular surgery. These tests have not been shown to be effective prognostic indicators for patients having nonvascular surgery. No studies of angiography for risk prediction have been reported. Decision analyses and retrospective series suggest that the risks incurred by doing coronary angiography and revascularization before elective surgery outweigh the benefits. Prospective, controlled studies of coronary revascularization are lacking. Evidence from a randomized, controlled trial has shown a survival benefit with the perioperative use of beta-blockers in patients at risk for coronary artery disease. CONCLUSIONS: Evaluation of all surgical patients by use of clinical indices is recommended. Low-risk patients need no further evaluation before surgery. High-risk patients need optimal management of their high-risk problems, including (if appropriate) beta-blocker use, and may need to have their elective procedures canceled. Intermediate-risk patients probably benefit from further noninvasive stress testing, especially if they are having vascular surgery. Further clinical trials are needed for most areas of concern.     

Gaucher disease: assessment of skeletal involvement and therapeutic responses to enzyme replacement

The clinical, pathological and radiological manifestations of type 1 Gaucher disease and the role of imaging techniques such as CT, MRI and sulfur-colloid scintigraphy in the management of these patients is discussed. MRI appears to be the most valuable imaging technique for evaluating bone marrow involvement and detecting disease activity. MRI is also useful in assessing therapeutic responses to enzyme replacement therapy.     

Complete transposition of great vessels: clinical aspects, diagnosis, echocardiography

The paper reports the study of 12 patients admitted to hospital between January 1992 and October 1994. The patients were almost all neonates. Clinical, electrocardiographic and radiological data led to the suspected diagnosis of complete transposition of the great vessels. 2-D echocardiography consistently showed the parallel disposition of great vessels, the anterior position of the aorta compared to the pulmonary artery, the origin of the pulmonary artery from the left ventricle and the aorta from the right ventricle, all of which are the anatomopathological characteristics of this congenital cardiopathy, together with associated malformations, thus enabling a certain diagnosis to be made. The authors underline the value of echocardiography in the diagnosis of complete transposition of the great vessels.     

Diagnosis of sclerotic arterial diseases of the lower limbs

Diagnosis and treatment of lower limbs arterial disease do not depend exclusively on functional staging. Fontaine's classification has to be replaced by a classification based on clinical and complementary investigations to understand better the evolutive risks, and guide management. Distal systolic pressures along with symptom analysis and clinical examination allow a more precise diagnosis. Complementary investigations aim at assessing, the localization and extension of atherosclerotic lesions; the severity of tissue ischaemia. The concept of critical ischaemia has tried to insure greater diagnostic homogeneity for patients with rest ischaemia. The role of complementary investigation can only increase for the diagnosis and management sclerotic arterial disease. The detection of the other localizations of atherosclerosis and arteriosclerosis are essential to prevent complications which are responsible for the prognosis of the disease. Sclerotic arterial diseases represent approximately 90 to 95% of the causes of arterial diseases. Identification of these other causes remains, however, essential as it can greatly influence prognosis.     

Safety and effectiveness of placement of pacemaker and defibrillator leads in the axillary vein guided by contrast venography

OBJECTIVE: To identify subjects with univentricular idiopathic structural and/or functional myocardial disorders (as defined) and to describe the characteristic features. DESIGN: Over a period of 4 years, 1993-1996, all adult subjects were obtained consecutively from a centralised referral cardiological service. The subjects had to fulfil a set of formulated diagnostic criteria for each isolated type of univentricular disease-symptomatic or asymptomatic. The subjects were diagnosed on the basis of clinical features, supported by electrocardiographic, radiological and echocardiographic evidence of lone ventricular disease, with a further definition of abnormalities based on appropriately selected standard left and right heart assessments, inter alia: (i) cardiac catheterisation, including coronary arteriography and pulmonary angiography; (ii) radio-isotope studies-mibiscan; (iii) ventilation perfusion scan; and (iv) laboratory tests to identify likely cause(s) of diffuse myocardial damage as well as to recognise nonspecific effects of tissue damage and organ dysfunction. SETTING: A referral cardiological service of a tertiary academic hospital, which provides a consultative service for inpatients and ambulatory cases. All subjects were studied on admission to hospital. PARTICIPANTS: A set of criteria was formulated for each category of lone ventricular myopathy. A total of 30 patients were thus identified and included in the study-men and women ranging in age from 18 years to 84 years, with an average of 48 years. All were investigated after admission to hospital by means of a detailed set of investigations that rigorously excluded overt or occult causes of diffuse myocardial damage and any severe myocardial dysfunction secondary to haemodynamic conditions. Seven patients with significant coronary artery disease were excluded. Any subject with pulmonary or systemic hypertension was also excluded. MAIN OUTCOME MEASURES: Total number of patients, number of patients in each subgroup were analysed by age, sex, clinical features, and by special investigation. The mode of presentation and electrocardiographic features were analysed separately. RESULTS: Twenty-two left ventricular and 8 right ventricular cases of lone ventricular cardiomyopathy were diagnosed. All but 1 patient with right ventricular disease were symptomatic and 5 subjects with left ventricular myopathy were incidentally discovered. There were 17 men and 13 women in the series. Of the 8 patients with right ventricular disease, 6 were women, while of the 22 patients with left ventricular cardiomyopathy, 15 were men. CONCLUSION: The study supports the previously described existence of lone ventricular idiopathic cardiomyopathy. Further studies are, however, indicated in order to define its prevalence and nature more accurately, as well as to describe any relationship with univentricular cardiomyopathies, and define the characteristics of each category and the possible evolutionary patterns. Right ventricular cardiomyopathy is a new entity which may pose difficult diagnostic challenges, while left ventricular disease is generally accepted as a stage in the clinical spectrum of classic idiopathic dilated cardiomyopathy.     

Assessment of acute myocardial necrosis after cardiopulmonary resuscitation and cardioversion by means of combined thallium-201/technetium-99m pyrophosphate tomography

Diagnosis of acute myocardial necrosis by means of conventional electrocardiographic criteria or the release of cardiac enzymes is often difficult or even impossible in patients with out-of-hospital cardiac arrest due to ventricular fibrillation with subsequent cardiopulmonary resuscitation including several DC countershocks. Simultaneous thallium-201/technetium-99m pyrophosphate (PYP) tomography was prospectively applied to 57 patients without typical clinical or electrocardiographic signs of acute myocardial infarction within 48 h after successful resuscitation from out-of-hospital cardiac arrest. Scintigraphic evidence of acute necrosis was present in 23/57 patients (40%). Increased 99mTc-PYP uptake in the pericardial tissue was found in 24 patients (42%). Maximal creatine kinase (CK) concentration was increased in 50/57 patients (88%). CK-MB activity averaged 68+/-52 U/l in patients with positive and 17+/-13 U/l in patients with negative tomograms (P<0.0005), demonstrating the validity of 201Tl/99mTc-PYP tomography. It may be concluded that simultaneous 201Tl/99mTc-PYP tomography is a valuable tool for evaluation of myocardial necrosis after cardiopulmonary resuscitation including DC countershock. Acute myocardial necrosis, as indicated by scintigraphy, represents a potential trigger for the occurrence of ventricular fibrillation. Therefore, 201Tl/99mTc-PYP tomography can be recommended in order to guide further diagnostic and therapeutic interventions in patients after cardiopulmonary resuscitation in whom the underlying cause of the occurrence of ventricular fibrillation is obscure.     

High-resolution electrocardiography in acute myocardial infarct

The author starts by highlighting the importance of risk stratification in patients who have survived a myocardial infarction. High resolution electrocardiography, also called signal-averaged electrocardiography (SAECG), appears in this setting as a diagnostic tool that, by providing important information about the way the intraventricular conduction of the electrical impulse is made, contributes to the characterization of the arrhythmogenic substrate, which is the basis of ventricular tachycardia and fibrillation. By resorting to the averaging of the electrocardiographic signal, SAECG enables us to detect ventricular late potentials whenever the analysis of that signal is made in time-domain. Further details, which will enrich the information on ventricular activation, can be obtained if the analysis is made in the frequency-domain (spectral analysis). The importance of detecting abnormalities in the SAECG recordings lies in the fact that those abnormalities are related to the occurrence of ventricular tachycardia and fibrillation, which are responsible for arrhythmic death. After referring to the criteria of positivity of SAECG and its reproducibility, the author approaches the most important part of the paper: the clinical applications of SAECG. After focusing on the interest of the method in noncoronary conditions, its usefulness in patients with acute myocardial infarction is pointed out. The author then mentions the prevalence of abnormalities in SAECG in patients with acute myocardial infarction and emphasizes the interest of the method in risk stratification. The author then presents the results of his Group in what concerns prevalence and prognosis. Finally, the author refers to the application of SAECG in other forms of coronary artery disease besides myocardial infarction.     

Fibromyalgia and chronic fatigue syndrome: similarities and differences

CFS and FM are clinical conditions characterized by a variety of nonspecific symptoms including prominent fatigue, myalgia, and sleep disturbances. There are no diagnostic studies or widely accepted, pathogenic, explanatory models for either illness. Despite remarkably different diagnostic criteria, CFS and FM have many demographic and clinical similarities. More specifically, few differences exist in the domains of symptoms, examination findings, laboratory tests, functional status, psychosocial features, and psychiatric disorders. FM appears to represent an additional burden of suffering among those with CFS, however, underscoring the importance of recognizing concurrent CFS and FM. Further clarification of the similarities (and differences) between CFS and FM may be useful in studies of prognosis and help define subsets of patients who may benefit from specific therapeutic interventions.     

Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adults

BACKGROUND: Chronic idiopathic intestinal pseudo-obstruction, a syndrome of ineffectual motility due to a primary disorder of enteric nerve or muscle, is rare. AIMS: To determine the clinical spectrum, underlying pathologies, response to treatments, and prognosis in a consecutive unselected group of patients. METHODS: Cross sectional study of all patients with clinical and radiological features of intestinal obstruction in the absence of organic obstruction, associated with dilated small intestine (with or without dilated large intestine), being actively managed in one tertiary referral centre at one time. RESULTS: Twenty patients (11 men and nine women, median age 43 years, range 22-67) fulfilled the diagnostic criteria. Median age at onset of symptoms was 17 years (range two weeks to 59 years). Two patients had an autosomally dominant inherited visceral myopathy. Major presenting symptoms were pain (80%), vomiting (75%), constipation (40%), and diarrhoea (20%). Eighteen patients required abdominal surgery, and a further patient had a full thickness rectal biopsy. The mean time interval from symptom onset to first operation was 5.8 years. Histology showed visceral myopathy in 13, visceral neuropathy in three, and was indeterminate in three. In the one other patient small bowel motility studies were suggestive of neuropathy. Two patients died within two years of symptom onset, one from generalised thrombosis and the other from an inflammatory myopathy. Of the remaining 18 patients, eight were nutritionally independent of supplements, two had gastrostomy or jejunostomy feeds, and eight were receiving home parenteral nutrition. Five patients were opiate dependent, only one patient had benefited from prokinetic drug therapy, and five patients required formal psychological intervention and support. CONCLUSIONS: In a referral setting visceral myopathy is the most common diagnosis in this heterogeneous syndrome, the course of the illness is usually prolonged, and prokinetic drug therapies are not usually helpful. Ongoing management problems include pain relief and nutritional support.     

Hospital mortality in ischemia: influence of two vascular risk factors

INTRODUCTION: Acute cerebrovascular disease is a serious neurological problem. Mortality is between 6% and 30%. Most studies are in agreement that advanced age, type of ictus, size of lesion and clinical deterioration are factors determining mortality. However, its relationship to vascular risk factors is not completely clear. OBJECTIVE: To analyze the repercussion of different vascular risk factors on mortality during the acute phase of ictus. PATIENTS AND METHODS: We studied all patients with ictus admitted to the Servicio de Neurología of the Hospital Xeral de Galicia de Santiago de Compostela over a period of 3 years (n = 915). We recorded vascular risk factors and analyzed the causes of mortality whilst in hospital. RESULTS: Hospital mortality due to ictus was 16.8%. Mortality was 14.5% in cerebral infarct, 23.2% in intracerebral haemorrhage and 19.4% in subarachnoid haemorrhage. A total of 20.8% of the patients died of neurological causes, 24.7% of vascular causes, 26% due to infection and of uncertain causes in the remaining 28.6%. There was a neurological cause of death in 46.1% of the patients with subarachnoid haemorrhage, 25.5% with intracerebral haemorrhage and 14.8% with cerebral infarcts. Vascular risk factors associated with greater mortality were age (p < 0.001) and a history of cardiopathy (p < 0.05). CONCLUSIONS: Vascular risk factors which indicated worse prognosis were: age, type of ictus and a history of cardiopathy.     

A comprehensive, interdisciplinary approach to the care of the institutionalized person with epilepsy

The methods of assessing the diagnostic usefulness of exercise testing in detecting coronary obstructive disease are examined. The limitations of long-term clinical follow-up and coronary angiography as standards for the determination of disease are described as are the effects of test methodology, criteria for positivity, prevalence of disease in the study population, reliability of the standard and recognition of false positive and false negative results in determining reliability of such testing. High values were found for sensitivity and specificity for exercise testing of patients with coronary artery disease referred for consultation to medical center cardiology services. Maximal exercise testing and consideration of symptomatic and hemodynamic as well as electrocardiographic criteria for identification of myocardial ischemia are helpful in improving reliability. Diagnostic accuracy is greatly enhanced by recognition of known causes for false positive and false negative results.     

Modulation of intracellular calcium in human neutrophils by peripheral benzodiazepine receptor ligands

Persistent disabling groin pain in an active sportsman is a frustrating diagnostic and management problem for both the athlete and physician. After clinical examination and investigation there remains a group of patients who have unexplained groin pain, and may undergo lengthy periods of conservative management with numerous radiological investigations. Here we highlight such a case.